Original article

Vol. 22 No. 1 (2024): Blood Transfusion 1-2024 (January-February)

Longitudinal prospective comparison of pancreatic iron by magnetic resonance in thalassemia patients transfusion-dependent since early childhood treated with combination deferiprone-desferrioxamine vs deferiprone or deferasirox monotherapy

Authors

Key words: thalassemia, chelating agents, magnetic resonance imaging, iron overload, pancreas
Publication Date: 2023-02-24

Abstract

Background - In transfusion-dependent thalassemia patients who started regular transfusions in early childhood, we prospectively and longitudinally evaluated the efficacy on pancreatic iron of a combined deferiprone (DFP) + desferrioxamine (DFO) regimen versus either oral iron chelator as monotherapy over a follow-up of 18 months.
Materials and methods - We selected patients consecutively enrolled in the Extension-Myocardial Iron Overload in Thalassemia network who received a combined regimen of DFO+DFP (N=28) or DFP (N=61) or deferasirox (DFX) (N=159) monotherapy between the two magnetic resonance imaging scans. Pancreatic iron overload was quantified by the T2* technique.
Results - At baseline no patient in the combined treatment group had a normal global pancreas T2* (≥26 ms). At follow-up the percentage of patients who maintained a normal pancreas T2* was comparable between the DFP and DFX groups (57.1 vs 70%; p=0.517).
Among the patients with pancreatic iron overload at baseline, global pancreatic T2* values were significantly lower in the combined DFO+DFP group than in the DFP or DFX groups. Since changes in global pancreas T2* values were negatively correlated with baseline pancreas T2* values, the percent changes in global pancreas T2* values, normalized for the baseline values, were considered. The percent changes in global pancreas T2* values were significantly higher in the combined DFO+DFP group than in either the DFP (p=0.036) or DFX (p=0.030) groups.
Discussion - In transfusion-dependent patients who started regular transfusions in early childhood, combined DFP+DFO was significantly more effective in reducing pancreatic iron than was either DFP or DFX.

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Authors

Paolo Ricchi - U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

Antonella Meloni - Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy; U.O.C. Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Laura Pistoia - Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Maria R. Gamberini - Unità Operativa di Day Hospital della Talassemia e delle Emoglobinopatie, Dipartimento della Riproduzione e dell’Accrescimento, Azienda Ospedaliero-Universitaria "S. Anna", Cona (FE), Italy

Liana Cuccia - Unità Operativa Complessa Ematologia con Talassemia, ARNAS Civico “Benfratelli-Di Cristina”, Palermo, Italy

Massimo Allò - Ematologia Microcitemia, Ospedale San Giovanni di Dio-ASP Crotone, Crotone, Italy

Maria C. Putti - Dipartimento della Salute della Donna e del Bambino, Clinica di Emato-Oncologia Pediatrica, Azienda Ospedaliero-Università di Padova, Padua, Italy

Anna Spasiano - U.O.S.D. Malattie Rare del Globulo Rosso, Azienda Ospedaliera di Rilievo Nazionale “A. Cardarelli”, Naples, Italy

Rosamaria Rosso - Unità Operativa Talassemie ed Emoglobinopatie Azienda Ospedaliero-Universitaria Policlinico “Vittorio Emanuele”, Catania, Italy

Valerio Cecinati - Struttura Semplice di Microcitemia, Ospedale “SS. Annunziata” ASL Taranto, Taranto, Italy

Riccardo Righi - Diagnostica per Immagini e Radiologia Interventistica, Ospedale del Delta, Lagosanto, Italy

Stefania Renne - Struttura Complessa di Cardioradiologia-UTIC, Presidio Ospedaliero “Giovanni Paolo II”, Lamezia Terme (CZ), Italy

Giuseppe Peritore - Unità Operativa Complessa di Radiologia, ARNAS Civico “Benfratelli-Di Cristina”, Palermo, Italy

Antonino Vallone - Reparto di Radiologia, Azienda Ospedaliera "Garibaldi" Presidio Ospedaliero Nesima, Catania, Italy

Vincenzo Positano - Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy; U.O.C. Bioingegneria, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Emilio Quaia - Institute of Radiology, Department of Medicine, University of Padua, Padua, Italy

Filippo Cademartiri - Department of Radiology, Fondazione G. Monasterio CNR-Regione Toscana, Pisa, Italy

Alessia Pepe - Institute of Radiology, Department of Medicine, University of Padua, Padua, Italy

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